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9 - MALT lymphoma and other marginal zone lymphomas

from Part II - LYMPHOMA SUBTYPES

Published online by Cambridge University Press:  05 March 2010

By
Emanuele Zucca ,
Francesco Bertoni ,
Andrew Wotherspoon ,
Andreas Rosenwald  and
German Ott
Edited by
Robert Marcus ,
John W. Sweetenham  and
Michael E. Williams
Emanuele Zucca
Affiliation:
Lymphoma Unit Oncology, Institute of Southern Switzerland, Ospedale San Giovanni 6500, Bellinzona, Switzerland
Francesco Bertoni
Affiliation:
Functional Genomics Unit, Laboratory of Experimental Oncology, Oncology, Institute of Southern Switzerland, 6500, Bellinzona, Switzerland
Andrew Wotherspoon
Affiliation:
Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK
Andreas Rosenwald
Affiliation:
Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
German Ott
Affiliation:
Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
Robert Marcus
Affiliation:
Addenbrooke's NHS Foundation Trust, Cambridge
John W. Sweetenham
Affiliation:
Case Western Reserve University, Ohio
Michael E. Williams
Affiliation:
University of Virginia
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Summary

EXTRANODAL MARGINAL ZONE B-CELL LYMPHOMA (MALT LYMPHOMA): CLINICAL FEATURES

Lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) comprises about 7–8% of all non-Hodgkin's lymphomas. It is a neoplasm of adults with a median age at presentation of about 60 years and with a slightly higher proportion of females than males. The presenting symptoms are essentially related to the primary location. Few patients present with elevated lactate dehydrogenase (LDH) or β2 microglobulin levels. Constitutional B symptoms are extremely uncommon. MALT lymphoma usually remains localized for a prolonged period within the tissue of origin, but dissemination to multiple sites is not uncommon and has been reported in up to one-quarter of cases, with either synchronous or metachronous involvement of multiple mucosal sites or non-mucosal sites such as bone marrow, spleen or liver. Regional lymph nodes can also be involved. Bone-marrow involvement is reported in up to 20% of cases. The stomach is the commonest localization, representing about one-third of the cases. Other typical presentation sites include the salivary glands, the orbit, the thyroid and the lung; the frequency at different organs is shown in Table 9.1.

Within the stomach, MALT lymphoma is often multifocal, possibly explaining the reports of relapses in the gastric stump after surgical excision. Gastric MALT lymphoma can often disseminate to the small intestine and to the splenic marginal zone. Concomitant GI and non-GI involvement can be detected in approximately 10% of cases. Disseminated disease appears to be more common in non-GI MALT lymphomas.

Type
Chapter
Information
Lymphoma: Pathology, Diagnosis and Treatment , pp. 126 - 140
Publisher: Cambridge University Press
Print publication year: 2007

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References

Arcaini, L., Lazzarino, M., Colombo, N.et al. Splenic marginal zone lymphoma: a prognostic model for clinical use. Blood 107 (2006), 4643–4649.CrossRefGoogle ScholarPubMed
Berger, F., Felman, P., Thieblemont, C.et al. Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients. Blood 95 (2000), 1950–1956.Google ScholarPubMed
Bertoni, F. and Zucca, E.State-of-the-art therapeutics: marginal-zone lymphoma. J. Clin. Oncol. 23 (2005), 6415–6420.CrossRefGoogle ScholarPubMed
Bertoni, F., Conconi, A., Capella, C.et al. Molecular follow-up in gastric mucosa-associated lymphoid tissue lymphomas: early analysis of the LY03 cooperative trial. Blood 99 (2002), 2541–2544.CrossRefGoogle ScholarPubMed
Chen, L. T., Lin, J. T., Tai, J. J.et al. Long-term results of anti-Helicobacter pylori therapy in early-stage gastric high-grade transformed MALT lymphoma. J. Natl. Cancer Inst. 97 (2005), 1345–1353.CrossRefGoogle ScholarPubMed
Conconi, A., Cavalli, F. and Zucca, E. MALT lymphomas: the role of chemotherapy. In MALT Lymphomas, ed. Zucca, E. and Bertoni, F. (Georgetown, TX: Landes/Kluwer, 2004) pp. 99–103.Google Scholar
Copie-Bergman, C., Gaulard, P., Lavergne-Slove, A.et al. Proposal for a new histological grading system for post-treatment evaluation of gastric MALT lymphoma. Gut 52 (2003), 1656.CrossRefGoogle ScholarPubMed
Lecuit, M., Abachin, E., Martin, A.et al. Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N. Engl. J. Med. 350 (2004), 239–248.CrossRefGoogle ScholarPubMed
Martinelli, G., Laszlo, D., Ferreri, A. J.et al. Clinical activity of rituximab in gastric marginal zone non-Hodgkin's lymphoma resistant to or not eligible for anti-Helicobacter pylori therapy. J. Clin. Oncol. 23 (2005), 1979–1983.CrossRefGoogle ScholarPubMed
Mollejo, M., Camacho, F. I., Algara, P., Ruiz-Ballesteros, E., Garcia, J. F. and Piris, M. A.Nodal and splenic marginal zone B cell lymphomas. Hematol. Oncol. 23 (2005), 108–118.CrossRefGoogle ScholarPubMed
Pinotti, G., Zucca, E., Roggero, E.et al. Clinical features, treatment and outcome in a series of 93 patients with low-grade gastric MALT lymphoma. Leuk. Lymphoma 26 (1997), 527–537.CrossRefGoogle Scholar
Ruskone-Fourmestraux, A., Dragosics, B., Morgner, A., Wotherspoon, A. and Jong, D. Paris staging system for primary gastrointestinal lymphomas. Gut 52 (2003), 912–913.CrossRefGoogle ScholarPubMed
Thieblemont, C. and Coiffier, B. MALT lymphoma: sites of presentations, clinical features and staging procedures. In MALT Lymphomas, ed. Zucca, E. and Bertoni, F. (Georgetown, TX: Landes/Kluwer, 2004) pp. 60–80.Google Scholar
Thieblemont, C., Berger, F., Dumontet, C.et al. Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood 95 (2000), 802–806.Google ScholarPubMed
Thieblemont, C., Felman, P., Callet-Bauchu, E.et al. Splenic marginal-zone lymphoma: a distinct clinical and pathological entity. Lancet Oncol. 4 (2003), 95–103.CrossRefGoogle ScholarPubMed
Tsang, R. W. and Gospodarowicz, M. K.Radiation therapy for localized low-grade non-Hodgkin's lymphomas. Hematol. Oncol. 23 (2005), 10–17.CrossRefGoogle ScholarPubMed
Wenzel, C., Dieckmann, K., Fiebeger, W., Mannhalter, C., Chott, A. and Raderer, M.CD5 expression in a lymphoma of the mucosa-associated lymphoid tissue (MALT)-type as a marker for early dissemination and aggressive clinical behaviour. Leuk. Lymphoma 42 (2001), 823–829.CrossRefGoogle Scholar
Wotherspoon, A. C. and Savio, A. Molecular follow-up in gastric MALT lymphomas. In MALT Lymphomas, ed Zucca, E. and Bertoni, F. (Georgetown, TX: Landes/Kluwer, 2004) pp. 91–98.Google Scholar
Zucca, E. and Cavalli, F.Are antibiotics the treatment of choice for gastric lymphoma?Curr. Hematol. Rep. 3 (2004), 11–16.Google ScholarPubMed
Zucca, E., Bertoni, F., Roggero, E. and Cavalli, F.The gastric marginal zone B-cell lymphoma of MALT type. Blood 96 (2000), 410–419.Google ScholarPubMed
Zucca, E., Conconi, A., Pedrinis, E.et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood 101 (2003), 2489–2495.CrossRefGoogle ScholarPubMed

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  • MALT lymphoma and other marginal zone lymphomas
    • By Emanuele Zucca, Lymphoma Unit Oncology, Institute of Southern Switzerland, Ospedale San Giovanni 6500, Bellinzona, Switzerland, Francesco Bertoni, Functional Genomics Unit, Laboratory of Experimental Oncology, Oncology, Institute of Southern Switzerland, 6500, Bellinzona, Switzerland, Andrew Wotherspoon, Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, Andreas Rosenwald, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany, German Ott, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
  • Edited by Robert Marcus, John W. Sweetenham, Case Western Reserve University, Ohio, Michael E. Williams, University of Virginia
  • Book: Lymphoma: Pathology, Diagnosis and Treatment
  • Online publication: 05 March 2010
  • Chapter DOI: https://doi.org/10.1017/CBO9780511663369.010
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  • MALT lymphoma and other marginal zone lymphomas
    • By Emanuele Zucca, Lymphoma Unit Oncology, Institute of Southern Switzerland, Ospedale San Giovanni 6500, Bellinzona, Switzerland, Francesco Bertoni, Functional Genomics Unit, Laboratory of Experimental Oncology, Oncology, Institute of Southern Switzerland, 6500, Bellinzona, Switzerland, Andrew Wotherspoon, Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, Andreas Rosenwald, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany, German Ott, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
  • Edited by Robert Marcus, John W. Sweetenham, Case Western Reserve University, Ohio, Michael E. Williams, University of Virginia
  • Book: Lymphoma: Pathology, Diagnosis and Treatment
  • Online publication: 05 March 2010
  • Chapter DOI: https://doi.org/10.1017/CBO9780511663369.010
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • MALT lymphoma and other marginal zone lymphomas
    • By Emanuele Zucca, Lymphoma Unit Oncology, Institute of Southern Switzerland, Ospedale San Giovanni 6500, Bellinzona, Switzerland, Francesco Bertoni, Functional Genomics Unit, Laboratory of Experimental Oncology, Oncology, Institute of Southern Switzerland, 6500, Bellinzona, Switzerland, Andrew Wotherspoon, Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, Andreas Rosenwald, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany, German Ott, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
  • Edited by Robert Marcus, John W. Sweetenham, Case Western Reserve University, Ohio, Michael E. Williams, University of Virginia
  • Book: Lymphoma: Pathology, Diagnosis and Treatment
  • Online publication: 05 March 2010
  • Chapter DOI: https://doi.org/10.1017/CBO9780511663369.010
Available formats
×